Spontaneous bleeding of the inferior pancreatic-duodenal artery in median arcuate ligament syndrome: do not miss the diagnosis

Abstract Median arcuate ligament syndrome (MALS) is a rare condition in which the median arcuate ligament (MAL) causes compression of the coeliac trunk. The chronic compression leads to coeliac trunk luminal narrowing and reduced blood supply to the abdominal splanchnic organs with possible local complications such as pseudo-aneurysms and spontaneous bleeding. Its incidence is probably underestimated due to the poor availability of color Doppler ultrasonography (CD-US), especially in an Emergency Department (ED) setting. A 44-year old woman presented to Pisa University Hospital ED with acute abdominal pain. An abdominal ultrasonography scan (US scan) was performed showing the presence of free liquid in the Douglas pouch. The abdominal computed tomography scan (CT scan) highlighted the presence of a large mesenteric hematoma. A CD-US revealed a significant stenosis of the coeliac artery. A selective angiography confirmed the diagnosis of MALS with a pseudo-aneurysm of the inferior pancreatic-duodenal artery, which was successfully embolized.

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Median arcuate ligament syndrome and a laparoscopic coeliac trunk first approach: a literature review and a proposed algorithm

International Surgery Journal ◽

10.18203/2349-2902.isj20214016 ◽

2021 ◽

Vol 8 (10) ◽

pp. 3160

Author(s):

Ji Chen ◽

Anik Sarkar ◽

Mouhannad Jaber

Keyword(s):

Abdominal Pain ◽

Literature Review ◽

Rare Condition ◽

Recurrent Abdominal Pain ◽

Young Female ◽

Review Of The Literature ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Arcuate Ligament ◽

Coeliac Trunk

Median arcuate ligament syndrome (MALS) is a rare condition where chronic recurrent abdominal pain is related to the compressive effects of the median arcuate ligament on the coeliac artery. The mechanism behind this phenomenon is incompletely understood but thought to be both ischemic and neuropathic in nature. As a result, the management of this condition remains controversial. Furthermore, while there have been a variety of options both radiological and interventional described for the investigation of MALS, there has yet to be a consensus in how patients exhibiting symptoms should be assessed and worked up. In this article, we described a laparoscopic coeliac trunk first approach in a young female patient and following review of the literature, propose an algorithm that can be used in the assessment and management of suspected MALS.

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New perspective on median arcuate ligament syndrome. Case reports

Journal of Ultrasonography ◽

10.15557/jou.2021.0037 ◽

2021 ◽

Vol 21 (86) ◽

pp. e234-e236

Author(s):

Andrzej Smereczyński ◽

◽

Katarzyna Kołaczyk ◽

Radosław Kiedrowicz ◽

◽

...

Keyword(s):

Collateral Circulation ◽

Case Reports ◽

Causative Factor ◽

Standing Position ◽

Left Lobe ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Arcuate Ligament ◽

Coeliac Trunk ◽

New Perspective

A large group of patients with significant asymptomatic or low-symptomatic coeliac trunk stenosis require deeper consideration. On angiography, CT and MRI, 10–24% of examined patients are found to have their coeliac trunk compressed by the median arcuate ligament of the diaphragm. The associated median arcuate ligament syndrome, which is also called coeliac trunk compression syndrome or Dunbar syndrome, is rarely fully symptomatic. It is estimated that there are up to 7% of patients with such a clinical presentation. An asymptomatic or low-symptomatic course of the disease in patients with the syndrome is mainly explained by a developed collateral circulation, particularly involving the arterial arcades of the head of pancreas. In such cases, CT angiography detects collateral circulation in 22–69.6% of examined patients. The present authors often observed coeliac trunk blood flow to normalise in a standing position. According to them, the main causative factor for this phenomenon is the deflection of the coeliac trunk and its compression against the aorta by a lowered left lobe of the liver. The researchers observed it in many individuals; in this study, 5 cases are presented.

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Treatment of median arcuate ligament syndrome: outcome of laparoscopic approach

ABCD Arquivos Brasileiros de Cirurgia Digestiva (São Paulo) ◽

10.1590/0102-672020190001e1495 ◽

2020 ◽

Vol 33 (1) ◽

Cited By ~ 1

Author(s):

Julio Cezar Uili COELHO ◽

Andréa Virmond El HOSNI ◽

Christiano MarloPaggi CLAUS ◽

Yan Sacha Hass AGUILERA ◽

Gisele Pitrowsk ABOT ◽

...

Keyword(s):

Weight Loss ◽

Epigastric Pain ◽

Clinical Manifestations ◽

Laparoscopic Approach ◽

Rare Condition ◽

Laparoscopic Treatment ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Arcuate Ligament ◽

Operative Findings

ABSTRACT Background: Median arcuate ligament syndrome(MALS) is a rare condition thatmay cause significant clinical manifestations, including abdominal pain and weight loss. Its diagnosis may be difficult and very often delayed. The laparoscopic approach became the standard treatment of MALS. Aim: To assess the outcome of laparoscopic treatment in patients with MALS. Method: The data of sixpatients with MALS who were subjected to laparoscopic sectioning of the median arcuate ligament were retrospectively reviewed.The following data were evaluated: age, gender, clinical and diagnostic tests findings, ASA score, operative findings and complications, postoperative complications and mortality, hospital stay duration, and hospital readmission.The diagnosis of MALS was established by CT angiography and/or MR angiography. Results: There were four (66.7%) women and two (33.3%) men aged from 32 to 60 years. The main symptoms were epigastric pain (100%) and weight loss (66.7%). The findings of high-grade stenosis of the proximal celiac axis and poststenotic dilation confirmed on angiography confirmed the diagnosis in all patients. Surgical procedure was uneventful in all patients. The only postoperative complication was urinary retention that occurred in a male. At three-month follow-up, all patients were asymptomatic. Conclusion: Laparoscopic treatment of MALS is safe and effective in relieving the clinical manifestations of patients.

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Abdominal Pain Associated With Anorexia Nervosa and Median Arcuate Ligament Syndrome: A Rare Condition Shadowed by a Postoperative Conversion Disorder

Vascular and Endovascular Surgery ◽

10.1177/1538574420927533 ◽

2020 ◽

Vol 54 (6) ◽

pp. 528-531

Author(s):

Charles J. Fox ◽

Alex Morton ◽

Ashlie Watters ◽

Philip S. Mehler

Keyword(s):

Anorexia Nervosa ◽

Abdominal Pain ◽

Rare Condition ◽

Conversion Disorder ◽

Comorbid Conditions ◽

Successful Operation ◽

Unique Case ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Arcuate Ligament

Median arcuate ligament syndrome (MALS) is an often discounted and vexing condition that may go unnoticed in a population of patients with complex comorbid conditions or those with poor psychiatric health. We report a unique case of a patient with anorexia nervosa in whom the successful operation for MALS was briefly complicated by a postoperative conversion disorder.

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Median arcuate ligament syndrome presenting with unintentional weight loss and epigastric pain

International Surgery Journal ◽

10.18203/2349-2902.isj20194452 ◽

2019 ◽

Vol 6 (10) ◽

pp. 3839

Author(s):

Mohammed Taher Mujahid ◽

Virendra Kumar Soni ◽

Rahul Saini

Keyword(s):

Weight Loss ◽

Rare Case ◽

Surgical Intervention ◽

Epigastric Pain ◽

Rare Condition ◽

Celiac Artery ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Extrinsic Compression ◽

Arcuate Ligament

Median arcuate ligament syndrome is a rare condition characterized by extrinsic compression of celiac artery and celiac plexus by the median arcuate ligament. Patients typically present with chronic postprandial pain, nausea and occasionally, vomiting and weight loss. Treatment of this syndrome is laparoscopic or open surgical release of median arcuate ligament and gangliectomy. We report a rare case of median arcuate ligament syndrome in a patient who presented with abdominal pain and nausea. The patient was evaluated, investigated and planned for surgical intervention but the patient refused for treatment.

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Median arcuate ligament syndrome, a rare case of chronic abdominal pain

Italian Journal of Medicine ◽

10.4081/itjm.2018.1041 ◽

2018 ◽

Vol 12 (3) ◽

pp. 219-222

Author(s):

Fania Puccia ◽

Roberto Citarrella ◽

Filippo Alessandro Montalto ◽

Luigi Mirarchi ◽

Antonino Terranova ◽

...

Keyword(s):

Internal Medicine ◽

Abdominal Pain ◽

Color Doppler ◽

Celiac Artery ◽

Confirmatory Test ◽

Forced Expiration ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

External Compression ◽

Arcuate Ligament

The median arcuate ligament syndrome (MALS) is a rare disease characterized by abdominal pain caused by the external compression of the celiac artery by the median arcuate ligament. Surgical treatment is indicated, but given the non-specific symptoms, these patients are often hospitalized in the Departments of Internal Medicine where the diagnosis may be unknown. We present a case of MALS admitted to our Internal Medicine Division. An abdominal ultrasound in a woman with longstanding abdominal pain showed elevated celiac artery velocities during forced expiration. Computed tomography angiography (CTA) of the abdomen showed stenosis of the origin of the celiac artery and confirmed the diagnosis of MALS. MALS is a syndrome that has to be considered, especially in young women with abdominal pain of unclear etiology; evaluated by color Doppler ultrasound, in the presence of elevated hepatic artery velocities during forced expiration, the confirmatory test is CTA.

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Atypical Presentation of Median Arcuate Ligament Syndrome in the Emergency Department

Clinical Practice and Cases in Emergency Medicine ◽

10.5811/cpcem.2019.9.44075 ◽

2019 ◽

Vol 3 (4) ◽

pp. 413-416 ◽

Cited By ~ 1

Author(s):

Abby Sapadin ◽

Ryan Misek

Keyword(s):

Emergency Department ◽

Imaging Techniques ◽

Treatment Options ◽

Rare Condition ◽

Celiac Artery ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Celiac Artery Compression Syndrome ◽

Gi Symptoms ◽

Arcuate Ligament

Celiac artery compression syndrome, also called median arcuate ligament syndrome (MALS), is a rare condition in which the diaphragmatic crura compresses the celiac axis. This results in a constellation of primarily gastrointestinal (GI) symptoms including nausea, vomiting, postprandial abdominal pain, and weight loss. It is typically a diagnosis of exclusion and may be detected via several imaging techniques including ultrasound and computed tomography angiography. We present an atypical case of MALS detected in the emergency department (ED). We review the symptomatology, diagnostic workup, and treatment options here, as well as discuss implications concerning revisits to the ED for recurrent GI symptoms.

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Laparoscopic decompression as treatment for median arcuate ligament syndrome

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2015.0025 ◽

2015 ◽

Vol 97 (6) ◽

pp. e96-e99 ◽

Cited By ~ 16

Author(s):

M Rubinkiewicz ◽

PK Ramakrishnan ◽

BM Henry ◽

J Roy ◽

A Budzyski

Keyword(s):

Epigastric Pain ◽

Treatment Options ◽

Recurrent Abdominal Pain ◽

Resonance Imaging ◽

Median Arcuate Ligament ◽

Median Arcuate Ligament Syndrome ◽

Arcuate Ligament ◽

Coeliac Trunk ◽

Abdominal Bruit ◽

Pain Patients

Median arcuate ligament syndrome (MALS) is a rare disorder due to coeliac trunk compression by the median arcuate ligament, resulting in coeliac artery stenosis characterised by chronic, recurrent abdominal pain. Patients with MALS are often middle-aged females presenting with a triad of postprandial epigastric pain, weight loss and abdominal bruit. It is a diagnosis of exclusion and confirmed by computed tomography or magnetic resonance imaging. Laparoscopic or open surgical decompression are the only treatment options in MALS. We present two cases of MALS treated by laparoscopic decompression as well as a literature review on this treatment.

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